The clinical course of idiopathic pulmonary fibrosis (IPF) is difficult to predict, partly owing to its heterogeneity. Composite physiologic index (CPI) and gender-age-physiology (GAP) models are easy-to-use predictors of IPF progression. This study aimed to compare the predictive values of these two models. From 2003 to 2007, the Korean Interstitial Lung Disease (ILD) Study Group surveyed ILD patients using the 2002 ATS/ERS criteria. A total of 832 patients with IPF were enrolled in this study. CPI was calculated as follows: 91.0 - (0.65 × %DLCO) - [0.53 × %FVC + [0.34 × %FEV1. GAP stage was calculated based on gender (0-1 points), age (0-2 points), and two physiologic lung function parameters (0-5 points). The two models had similar significant predictive values for patients with IPF (p < 0.001). The area under the curve (AUC) was higher for CPI than GAP for prediction of 1-, 2-, and 3-year mortality in this study. The AUC was higher for surgically diagnosed IPF patients than for clinically diagnosed patients. However, neither CPI nor GAP yielded good predictions of outcomes; the AUC was approximately 0.61~0.65. Although both CPI and GAP stage are significantly useful predictors for IPF, they have limited capability to accurately predict outcomes.
Pediatric reconstruction using microsurgery is accepted normal practice, and the use of perforator flaps is slowly increasing. This study presents clinical work using various perforator free flaps by free style approach to reconstruct lower extremity soft tissue defects in pediatric patients and evaluates its efficacy. Between June 2002 and February 2011, 32 cases (mean age: 10.1 years) were reconstructed with free style perforator free flaps. Retrospective evaluations for flap survival, growth character, and other associated morbidities were performed. Flaps used in this series are anterolateral thigh (ALT) perforator, superficial circumflex iliac artery perforator (SCIP), upper medial thigh perforator, and posterior interosseous perforator free flaps. The free style approach for pedicle dissection was successful in all cases. Early postoperative complications were 15.6% from hematoma collection to partial loss of flap. Although there was no total loss in this series, one case needed additional flap coverage to cover the partial loss of the flap. The long-term follow-up showed contracture along the margin, with 16% needing a releasing procedure. Bone growth was not affected by flap contracture. The overall results show perforator flaps using the free style approach to be a reliable and feasible approach for lower extremity reconstruction in the pediatric population.
Introduction: Plasma mitochondrial DNA (mtDNA) is known as a predictor of all-cause mortality in idiopathic pulmonary fibrosis (IPF) patients. However, the relationship between plasma mtDNA and disease progression in IPF are not well defined. Aims: To investigate the role of plasma mtDNA as a biomarker for disease progression in IPF. Methods: Clinical data and plasma mtDNA levels at diagnosis were retrospectively analyzed in 363 IPF patients (mean age: 64.0 years, male: 78.5%). Plasma mtDNA was measured by real-time polymerase chain reaction and expressed as a copy number of the human nicotinamide adenine dinucleotide reduced (NADH) dehydrogenase 1 gene. Disease progression was defined as ≥ 10% decline in forced vital capacity (FVC) at six months Results: Median follow-up period was 45.8 months. The baseline levels of mtDNA showed significant correlation with body mass index (r=0.114, P=0.044), diffusing capacity (DLco) (r=0.203, P<0.001), the lowest oxygen saturation during 6-minute walk test (r=-0.139, P=0.015), and decline in FVC (r=-0.135, P=0.040) and DLco (r=-0.224, P<0.001) at six months. Disease progression was identified in 16.7% of 252 patients who have lung function data at 6 months. Progressed group showed a higher level of mtDNA than non-progressed group (249.9 vs. 145.3 copies/uL, P=0.038). Following covariates adjustments for age, sex, smoking, FVC and DLco (% predicted), the elevated mtDNA level (≥33.7 copies/uL) was an only independent predictor for disease progression in multivariate analysis (odds ratio, 2.006; 95% CI, 1.007-3.998; P=0.048). Conclusions: Our finding suggest that plasma mtDNA could be useful for predicting disease progression in IPF.
Abstract Background: Riociguat is a novel soluble guanylate cyclase stimulator, and has been widely used for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (CTEPH). Some studies found that riociguat had better effects on CTEPH and proved to be safe, but the results were not utterly consistent. Therefore, the purpose of this study was to comprehensively evaluate the efficacy and safety of riociguat in the treatment of CTEPH. Methods: Randomized controlled trials on riociguat for the treatment of CTEPH were searched through such electronic databases as PubMed, Embase, Cochrane Library, Web of Science, China national knowledge internet, and Wanfang. The outcomes included exercise capacity, pulmonary hemodynamics, and side effects. The fixed-effects or random-effects models were used to analyze the pooled data, and heterogeneity was assessed by the I 2 test. Results: Four studies involving 520 patients were included in this meta-analysis. Compared with the placebo group, riociguat significantly improved the hemodynamic indexes and increased 6-min walking distance ( P < .0001, standardized mean difference (SMD) = −0.24, 95%CI −0.35 to −0.12; P < .00001, SMD = 0.52, 95%CI 0.33 to 0.71), and decreased the Borg dyspnea score ( P = .002, SMD = −0.31, 95%CI −0.51 to −0.12). In addition, riociguat could also significantly reduce the living with pulmonary hypertension scores and increase the EQ-5D scores ( P = .01, SMD=−0.23, 95%CI −0.42 to −0.05; P < .00001, SMD = 0.47, 95%CI 0.27 to 0.66), but there was no significant difference in the change level of N-terminal pro-hormone B-type natriuretic peptide in patients with riociguat ( P = .20, SMD = −0.24, 95%CI −0.61 to −0.13). The common adverse events of riociguat were dyspepsia and peripheral edema, and no other serious adverse reactions were observed. Conclusions: We confirmed that riociguat had better therapeutic effects in improving the hemodynamic parameters and exercise capacity in patients with CTEPH without inducing serious adverse events. This will provide a reasonable medication regimen for the treatment of CTEPH.
Introduction: acute exacerbation (AEx) is the most lethal postoperative complication in idiopathic pulmonary fibrosis (IPF); however, predicting factors are not well defined. Aims: to investigate the value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) for predicting postoperative AEx in IPF Methods: We retrospectively analyzed 48 IPF patients (mean age: 67.8 years, male: 90%) who underwent 18F-FDG PET/CT for for cancer evaluation and performed thoracic surgery at Asan Medical Center, Seoul, South Korea. Standardized uptake value (SUV) was measured in fibrotic area and lesion-to-liver SUV ratio (SUVR) was also obtained. Results: Among 48 patients, 44 underwent surgery for lung cancer and 4 for lung biopsy. Within 30 days after surgery, AEx and death occurred in 6 (12.5%) and 7 (14.6%) patients, respectively. SUV (3.1±0.8 vs. 2.4±0.6, p=0.026) and SUVR (1.7±0.5 vs. 1.3±0.4, p=0.005) were significantly higher in patients with AEx compared to those without. SUV (odds ratio [OR], 3.709; p=0.041) and SUVR (OR, 20.592; p=0.017) were also predicting factors for AEx by univariate logistic analysis; however, any other factors including baseline lung function were not. On receiver operating characteristics curve analysis, SUV (area under the curve [AUC], 0.754; the best cut-off value, 2.44; p=0.010) and SUVR were significant predictors for AEx (AUC, 0.806; cut -off value, 1.35; p=0.007). Conclusions: Our findings suggest that 18F-FDG PET/CT may be useful to predict AE after operation in patients with IPF and SUVR seems to be superior to SUV
A 19-year-old male patient was referred to our hospital for recurrent pneumothorax. He previously experienced seven episodes of pneumothorax refractory to conventional treatment including pleurodesis and wedge resection. On admission, chest computed tomography scan showed multiple cystic lesions with surrounding ground-glass opacities and several nodules in both lungs. Detailed history revealed that the patient experienced haemoptysis whenever pneumothorax developed and had a family history of sudden death. Physical examination showed large eyes with conjunctival injection, hypermobile joints, and hyper-extensive and easily bruised skin. All these findings led to the suspicion of vascular Ehlers-Danlos syndrome (EDS). Genetic testing for the diagnosis of vascular EDS was performed and a heterozygous mutation in
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